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Biomedical subjects

B Z Morgenstern

Publications and source records attributed to B Z Morgenstern.

At least 19 recordsLinked to original sources

Wilms' tumor and neuroblastoma.

Significant differences exist between the European and North American treatment protocols for Wilms' tumor and neuroblastoma. There are variations in biopsy technique, timing and extent of initial surgery, chemotherapy protocols and dosage routines, as well as the type of salvage therapy. With the consolidation of the two major North American study groups into a single entity (Children's Oncology Group), the European and North American study groups represent the only remaining large-scale venues for treatment comparison. It is important to study and understand the variation in treatment protocols in order to maintain an open forum of scientific investigation that will lead to improving the care and outcome of children with cancer. It is anticipated that the unification of the North American groups will lead to greater interest and scientific cooperation with the European study group. This paper will serve as a forum for such a discussion at a local level.

Clinical Protocols↗

Disseminated varicella infection in pediatric renal transplant recipients treated with mycophenolate mofetil.

BACKGROUND: Mycophenolate mofetil (MMF) is a new immune suppressive agent, effective in the prevention of acute rejection after renal transplantation. METHODS: The study was a retrospective review of records of pediatric renal transplant recipients from 1985 to the present. RESULTS: Since October 1995, the immune suppression protocol for pediatric renal transplant recipients at Mayo Eugenio Litta Children's Hospital has included MMF, prednisone, and cyclosporine A. During that time, 19 children and adolescents have received renal allografts, 17 of whom were seropositive for varicella antibody before transplantation, while 2 were seronegative. Varicella infection occurred in 3 of 19 patients (15.8%), all 3 of whom had serologically documented immunity to varicella virus before transplantation. All episodes occurred within 12 months of transplantation. All had generalized vesicular lesions without dermatomal distribution. None of the patients developed fever, respiratory, mucocutaneous, or central nervous system manifestations. All were managed with oral acyclovir, and had an uncomplicated recovery without neuralgia. By contrast, of 74 consecutive patients transplanted before use of MMF, only 1 patient (1.4%) had varicella infection after transplantation (P=0.026). CONCLUSION: The enhanced immunosuppression achieved with MMF appears to be associated with increased susceptibility to varicella infection.

Adolescent↗

Hypertension after surgical management of renal duplication associated with an upper pole ureterocele.

PURPOSE: Duplicated upper pole systems associated with a ureterocele frequently have areas of segmental renal dysplasia. Since dysplasia has been related to the development of renin mediated hypertension, we hypothesized that preservation of functional upper pole moieties may result in an increased incidence of high blood pressure. MATERIALS AND METHODS: We evaluated 115 patients with a history of renal duplication and an ipsilateral upper pole ureterocele. Patients were separated into group 1-nonfunctional upper pole managed by partial nephrectomy, group 2-functional upper pole moiety managed by partial nephrectomy and group 3-functional upper pole moiety managed by a nephron sparing procedure. All charts were reviewed for hypertension, febrile urinary tract infection, vesicoureteral reflux and renal scarring. RESULTS: At a median followup age of 15 years (range 1 to 33) hypertension developed in 13 of the 115 patients (11%), including 8 of the 87 (9%) with nonfunctional upper pole systems managed by partial nephrectomy, 1 of the 12 (8%) with a functional upper pole moiety managed by partial nephrectomy and 4 of the 16 (25%) with a functional upper pole system managed by an upper pole salvage procedure. Statistical evaluations failed to reveal any relationship between hypertension and preservation of the upper pole system or between hypertension and vesicoureteral reflux. Rather, elevation in blood pressure was found to be related to development of a renal scar after a febrile urinary tract infection. CONCLUSIONS: Hypertension in patients with a history of renal duplication associated with a ureterocele is directly related to renal scarring.

Child↗

An unusual renal complication in a patient with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a common 'rare' disorder with a reported incidence of 1/15,000 to 1/20,000 in newborns. Skeletal abnormalities in this condition are so striking that its equally important and diverse extraskeletal manifestations frequently remain unnoticed. Sensorineural deafness, blue sclerae, myopia, easy bruisability, dental anomalies, and floppy mitral valves are some of its more frequently reported extraskeletal features. Other less common features include pulmonary hypoplasia, joint contractures, hydrocephalus, and osteogenic sarcoma. Our patient, a 16-year-old boy, developed a previously unreported complication: chronic renal failure, which on subsequent evaluation was attributed to obstructive uropathy secondary to bony pelvic outlet deformities.

Acetabulum↗

Urinary tract anomalies detected by prenatal ultrasound examination at Mayo Clinic Rochester.

OBJECTIVE: To determine the frequency of fetal urinary tract anomalies and to characterize the types of such abnormalities detected on ultrasonography and the outcome of affected patients during a 15-year period at our institution. DESIGN: We retrospectively reviewed the findings on maternal prenatal ultrasound examinations and the postnatal medical records of 56 children with urinary tract abnormalities detected by prenatal ultrasound examination at Mayo Clinic Rochester from November 1979 to June 1994. RESULTS: Of the 56 children, 18 (32%) had severe urinary tract anomalies in conjunction with oligohydramnios, pulmonary hypoplasia, and perinatal death (Potter's syndrome). The other 38 infants had various urinary tract abnormalities--most commonly, isolated hydronephrosis and multicystic dysplasia of the kidney. Six of the 38 children had more than one renal abnormality detected prenatally. Reflux was noted in association with prenatally detected urinary tract abnormalities in 4 of 32 newborns (12%) who underwent voiding cystourethrography. In fetuses with normal amniotic fluid volume, the perinatal outcome was good. Children with lower urinary tract obstruction had evidence of more severe renal dysfunction than did those with involvement at more proximal levels. The presence or absence of urinary tract obstruction postnatally could not be determined reliably on the basis of prenatal ultrasound appearance. CONCLUSION: In this study, more than half of all prenatally detected urinary tract abnormalities were isolated hydronephrosis or multicystic dysplasia of the kidney. Postnatal renal function could not be reliably predicted on the basis of prenatal ultrasound findings.

Female↗

Effect of cyclosporine A on long-term allograft function in pediatric renal transplant recipients.

There have been concerns regarding long-term adverse effects of cyclosporine A (CSA) on renal allograft function. In a retrospective study, we compared long-term allograft function up to 70 months after renal transplantation in pediatric recipients treated with and without CSA, using iothalamate clearance to assess glomerular filtration rate. Patients received CSA, prednisone, and azathioprine (CSA group, n = 16) or prednisone and azathioprine alone (Pred/AZA, n = 11). At 48 months post transplant, the iothalamate clearances (mean +/- SD) were 57.9 +/- 26.8 ml/min per 1.73 m2 in the CSA group and 68.5 +/- 20.2 in the Pred/AZA group (P > 0.05). The mean of the slopes of individual iothalamate clearances versus time during the first 70 months following transplantation were -0.156 in the CSA group and 0.095 in the Pred/AZA group. Neither slope was statistically different from zero. These data suggest that allograft function is not significantly depressed by CSA at 48 months post transplantation and that there is no greater rate of decline in allograft function up to 70 months post transplantation in patients receiving CSA when compared with the AZA/Pred group.

Adolescent↗

Growth of children following the initiation of dialysis: a comparison of three dialysis modalities.

Maintenance dialysis usually serves as an interim treatment for children with end-stage renal disease (ESRD) until transplantation can take place. Some children, however, may require dialytic support for an extended period of time. Although dialysis improves some of the problems associated with growth failure in ESRD (acidosis, uremia, calcium, and phosphorus imbalance), many children continue to grow poorly. Therefore, three different dialysis modalities, continuous ambulatory peritoneal dialysis (CAPD), cycler/intermittent peritoneal dialysis (CPD), and hemodialysis (HD), were evaluated with regard to their effects on the growth of children initiating dialysis and remaining on that modality for 6-12 months. Growth was best for children undergoing CAPD when compared with the other two modalities with regard to the following growth parameters: incremental height standard deviation score for chronological age [-0.55 +/- 2.06 vs. -1.69 +/- 1.22 for CPD (P < 0.05) and -1.80 +/- 1.13 for HD (P < 0.05)]; incremental height standard deviation score for bone age [-1.68 +/- 1.71 vs. -2.45 +/- 1.43 for CPD (P = NS) and -2.03 +/- 1.28 for HD (P = NS)]; change in height standard deviation score during the dialysis period [0.00 +/- 0.67 vs. -0.15 +/- .29 for CPD (P = NS) and -0.23 +/- .23 for HD (P = NS)]. The reasons why growth appears to be best in children receiving CAPD may be related to its metabolic benefits: lower levels of uremia, as reflected by the blood urea nitrogen [50 +/- 12 vs. 69 +/- 16 mg/dl for CPD (P < 0.5) and 89 +/- 17 for HD (P < 0.05)], improved metabolic acidosis, as indicated by a higher serum bicarbonate concentration [24 +/- 2 mEq/l vs. 22 +/- 2 for CPD (P < 0.05) and 21 +/- 2 for HD (P < 0.05)]. In addition, children undergoing CAPD receive significant supplemental calories from the glucose absorbed during dialysis. CAPD, and possibly, other types of prolonged-dwell daily peritoneal dialysis appear to be most beneficial for growth, which may be of particular importance for the smaller child undergoing dialysis while awaiting transplantation.

Adolescent↗

Hypertension in pediatric patients: current issues.

OBJECTIVE: To present current information on the diagnosis, treatment, and epidemiologic features of hypertension in children. DESIGN: The guidelines published by the Task Force on Blood Pressure Control in Children in 1987 are critically evaluated, and more recent data are discussed. RESULTS: Prevention of the consequences of hypertension necessitates recognition of the condition. Although hypertension in the pediatric age-group is an uncommon finding, it does exist. The diagnosis is based on careful technique, repetitive blood pressure measurements, and comparison with established norms. Normal blood pressure values for children have been derived from a series of large cross-sectional studies. Although they have certain shortcomings, they are useful. Height and weight are important determinants in the use of the normative data. The selection of a blood pressure cuff that completely encircles the arm is crucial. Tracking--the persistence of systolic and diastolic pressures at stable percentiles--is not a constant finding, but children at risk for development of hypertension as adults may be identifiable. As in the adult population, children with hypertension have no signs or symptoms that clearly distinguish primary from secondary hypertension. CONCLUSION: Treatment of children with hypertension should be individualized. Etiologic evaluation is probably best performed at referral centers.

Adolescent↗

Urinary oxalate and glycolate excretion patterns in the first year of life: a longitudinal study.

To determine the range of normal values, we studied urinary excretion of oxalate and glycolate prospectively and longitudinally in a cohort of 30 healthy term infants. Random urine samples were obtained at 2 days, 2 weeks, and 2, 4, 6, and 9 months of age. The results had a log-normal distribution. The mean oxalate/creatinine ratio, as determined by the oxalate oxidase method, was 0.08 (in milligrams of oxalate to milligrams of creatinine) with a normalized range of 0.02 to 0.31. The mean ratio when oxalates were measured by using the Olthuis assay was 0.13 (range 0.03 to 0.53). The mean glycolate/creatinine ratio was 0.07 (range 0.02 to 0.26). These values did not correlate with increasing age. The oxalate/creatinine ratios were higher in formula-fed infants than in those who were fed human milk (0.14 +/- 0.16 vs 0.08 +/- 0.04; p < 0.01).

Creatinine↗